OUR JOURNEYS

Caregiver story

Margaret Goodwin

My name is Margaret Goodwin and this is my story…..

When I was 25 years old, I married Steve Everett. We had two children together. In the spring of 1970, Steve got sick and my whole life changed. He began with what we thought was a cold in his chest. By May, Steve was coughing a lot and this was not getting any better, so he made a doctor’s appointment for the following day so he could go when he got off from work. He worked the midnight shift, and went to work that night knowing he would be seeing a doctor the next day. During his shift, he passed out, or had a syncopal spell, at work. He was taken by ambulance to the local hospital where he was admitted for several days. Because the doctors there could not figure out what was wrong with him, he was transferred to a larger hospital a few miles from our hometown. He was there a few more days, but continued to puzzle the doctors as to what this condition was. Still not knowing what to do or how to treat him, he was transferred to another hospital in Chattanooga, TN. There he underwent more testing until he was diagnosed with Primary Pulmonary Hypertension (PPH). The doctors told me that he had a serious heart and lung disease, that there was no cure for this and that he would likely only live about three to six months with this pulmonary hypertension. He spent most of his remaining days in the hospital with the exception of the 4 days he got to come home. He lived from early May to the 4th of August that year, which was approximately 3 months. Back in the ’70s, the only treatments available to treat PPH were blood thinners and heart pills. There were no transplants going on. With the limitations of treatment then, my husband Steve lost his life to PPH at age 27, dying only 3 months after diagnosis.

During his illness, I did not know what to do. I spent all the time I could with Steve, and was there with him during all three of his hospital stays, day and night. The children were young, ages 6 and 1, and I was not getting to spend much time at all with them throughout this time. They came to the hospital to visit, but could not stay there as I did. It was hard for me when Steve passed away, for I had the girls, not knowing how we would get by. A lot of friends and family supported us with phone calls and visits. They were there for me and the girls, even if I just needed to talk. I was back to work in a few weeks, and while it was hard for us, we did okay.

When my oldest daughter reached her early 20s, and had been married only a short time, she started getting short of breath. When she went to the doctor, she too was diagnosed with PPH. She didn’t work long before she had to quit her job as she could not tolerate the physical demands from her job. That was when the doctors told her they could put her on a transplant list for a heart and lung transplant, but also that if she did not have a transplant, she would not survive much longer. The doctors have come a long way with doing transplants and there are many new medications to keep transplant patients from rejecting the organs. We were still scared because no one else in our family had gone thru a transplant. The doctors told Pam (Everett), that she would need to raise $75,000 up front, for some of the expenses associated with transplant, before she could be actively listed for the actual transplant. This also was frightening, because what would happen if we could not raise the money? But people went to work and did all kinds of fundraising for her. She had only a month or so to raise the $75,000, but she raised the money and was put on the list.

It was November 1990 on Thanksgiving Day and we were having a big holiday dinner at my house. The day after Thanksgiving Pam and her husband came back to my house to eat leftovers for dinner. After eating, we were all going Christmas shopping. Just after Pam and her husband walked out of the house, Pam called back to say that the doctors had called and they had a lung that would be available for transplant. At first, they had told her that she would need a heart and lung, but then decided that she would only need a single lung transplant. We did not waste any time getting to Vanderbilt Hospital in Nashville, TN, in fact we made the drive in 2 ½ hours, not the usual three hours that it normally had taken.

When we arrived, we had numerous papers to sign and they asked Pam if she was ready for the surgery. She was having some second thoughts and even asked if she could change her mind, but then decided to have the transplant surgery. She was in the operating room for 12 hours, but it seemed like days as we sat waiting in the waiting room. The doctors came out from time to time to let us know how things were going. Fortunately the surgery went well. Shortly after the operation was completed, Pam took a turn for the worse. We thought we were going to lose her. Her kidneys were failing and her right lung collapsed. The doctors told us that they might have to put her on a dialysis machine if her kidneys did not start to function. But one of the doctors said that he would like to try one more medicine before going with the dialysis. And in no time at all, her kidneys started working again. She was in the hospital for about two months and then had to stay close to the hospital for another five or six weeks. We were in a motel a few blocks from the hospital, in case any went wrong, so that we could get to the hospital quickly for medical care. It was a stressful and difficult time, and a very long wait until a transplant was available, but it was well worth this time. Pam was the fourth lung transplant at Vanderbilt Hospital and Dr. William Frist, now a US senator, was her transplant surgeon. She is still doing well at what will be 16 years for her this November.

More of our family members have had PPH and have not lived to get a transplant. We have been told that this disease can skip generations but in our family, it has not done this. In 2000, my other daughter, Tonya Everett White was pregnant with her last baby. She began to have fainting spells and we thought it was just her pregnancy causing this. But as she neared her delivery date, she had some problems. A few weeks later, she was diagnosed with PPH. Again we went to Vanderbilt Hospital to see the doctors. Tonya was being treated by Dr. Ivan Robbins. He and his nurse, Cindy, worked very closely with us, and we talked on the phone with Cindy or another nurse almost every week. Tonya was on a lot of medicine to treat her PPH, including a medicine called Flolan, which must be given continuously via a pump. It has to be mixed every day also and can not be stopped of a person could die. The Flolan helped Tonya for a while, but then she started having marital problems which increased her stress level significantly. The stress only compounded the symptoms of the PPH. After a while Tonya was not responding as well to the Flolan. She had five children to care for, mostly on her own. She did a good job with the kids as she took them to school, to church and anywhere else they needed to go. She did this for a few months until she began to get more short of breath. When this occurred she was started on home oxygen. After this, she spent more and more time at home. I helped her as much as I could, visiting often and doing her housework or anything else that needed to be done, so she and the children could spend time together. They did homework or sat and watched TV with their mom. It was hard to see Tonya going down so fast. It got to the point that she was not sleeping due to the pain and discomfort she was having. She also had undergone a workup for lung transplantation and was listed at Vanderbilt awaiting a call, just as Pam had done. She and the children came to my house to live after it got so bad. Then one day as she was continuing to decline, we got the call from Vanderbilt saying that they had a set of lungs for her. We got to Vanderbilt and Tonya was placed in a room. We all hoped and prayed that she would be a match for the available organs. Just as they were taking her to the holding room outside of the operating room, the doctors came in and said, they were sorry, but it was not a match. Our hearts were broken, but unfortunately this happens sometimes and we had to accept that this time was just not meant to be Tonya’s turn. Hoping for another chance for her was all we could do.

But as she was at my house, all the days and nights ran together. Tonya couldn’t sleep at night, but could get up to do much thru the day either. We would take care of the children and get them settled in bed, then try to rest ourselves, but Tonya could not stay in bed for more than two hours. She would wake me up and say “Mom, I am sorry but my legs are killing me, can you do something?” I would sit up the rest of the night rubbing her legs to ease the pain. I would call the doctor and ask him if there was anything we could do. He said that Tonya was taking all the medications that he had to treat her. The pain is from the PPH and we hope to get some lungs for her soon.

On Easter Sunday 2003, our family was at my house for Easter dinner. Just as lunch was almost done, Tonya asked me to come to where she was. As I went to the living room where she lay on the couch, she looked at me with a look that I have never seen before. She said, “Mom, I am so sick and we need to go to Nashville.” I called Dr. Robbins and told him that I was bringing Tonya to the hospital, and he said he would reserve a room for her and call us back with the details. We rushed to finish eating dinner, packed a few things and were on our way to the hospital. Shortly after we arrived and got to the room, the staff were busy putting in IV’s and getting monitors hooked up to Tonya. Early the next morning (Monday), the doctor came in and said that he thought they had a set of lungs for Tonya. We were so surprised to get these so soon after coming to the hospital. That evening, she went to surgery and it seemed like it took hours before we got to see her. The surgery staff did keep us informed that she was doing okay. A few hours after she came out of surgery, Tonya took a turn for the worse. A lot of things went wrong for her and we lost her the following Wednesday. She was so sick and weak prior to the surgery that she did not tolerate the stresses an operation this big brought with it.

She left behind five children, two of which I am raising. It was just like starting over again. I care for Tonya’s two oldest daughters on a day to day basis. But as fate would have it, once again we must deal with PPH. Tonya’s fifteen year old daughter, Karista Middleton, was diagnosed with PPH in October of 2005. Dr. Jeffery Jennings in Knoxville, TN did the test and told us that the pressures in Karista’s lungs were high. The systolic pressure was in the high seventies and normal is 30 or less. He said that he would call Vanderbilt Children’s Hospital to make her an appointment. About two weeks later, she was seen by the doctors, had more testing done and was admitted to Children’s Hospital for a heart catheterization. Her pulmonary artery pressures were getting worse and were already in the high ’80s. Dr. Donald Moore is her physician at Vanderbilt and he started Karista on a combination of medicines including Revatio and Ventavis. Ventavis is a medication that you put in an inhaler and breathe in to your lungs directly. You must do these treatments every two to three hours, and Karista was doing eight treatments a day. She was on this medication for about nine months when the doctors decided to repeat her heart catheterization to measure her pulmonary artery pressures. This would tell them how well she was tolerating the medication and how much it was helping her. Unfortunately the pressures were even higher than before. The systolic pressure was nearly 100. Dr. Moore said that he would have a meeting with some other physicians to see what medicines to put Karista on to treat her condition more effectively. At this time Karista was put on Flolan, warfarin – a blood thinner, and she continues the revatio. She developed some stomach symptoms so she also started Ranitidine.

Karista is doing somewhat better since going on Flolan. I can see her doing more day by day. It is hard for me to again see this disease take a toll on our family. We have been told that the other children may have PPH at any time in their life and that we should watch them closely for symptoms. I so look forward to the day that there will be a cure for this ugly, horrible disease.

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